Patients with bicuspid aortic valves commonly require an intervention on their valve and/or aorta. Because of their heterogeneous presentations, recommendations for imaging surveillance and surgery timing are highly individualized. Critical points in care include time of diagnosis, transition from adolescent to adult medicine, and surgery referral. To better support patients with bicuspid aortic valves, we developed a comprehensive program that utilizes the multidisciplinary care team, complex interventions, and translational research protocols. We describe our program structure and experience with this common and sometimes challenging diagnosis.
Atrial fibrillation (AF) is the most common of all cardiac arrhythmias, affecting roughly 1% of the general population in the Western world. The incidence of AF is predicted to double by 2050. Most patients with AF are treated with oral medications and only approximately 4% of AF patients are treated with interventional techniques, including catheter ablation and surgical ablation. The increasing prevalence and the morbidity/mortality associated with AF warrants a more aggressive approach to its treatment. It is the purpose of this invited editorial to describe the past, present, and anticipated future directions of the interventional therapy of AF, and to crystallize the problems that remain.
Abstract Background Alkaptonuria is a rare metabolic disease that causes an increase in homogentisic acid (HGA) due to a lack of enzymatic activity. Commonly, accumulation of HGA presents with dark discoloration of skin and other tissues, also known as ochronosis. Additionally, alkaptonuria can result in other clinical manifestations, including arthritis and cardiac disease. This case highlights alkaptonuria-related cardiac disease and challenges that cardiac surgery teams may face when treating this patient population. Case summary A 62-year-old male with a history of alkaptonuria, Hodgkin’s lymphoma treated with chemoradiation, hypertension, and hyperlipidaemia originally presented with shortness of breath in the setting of known cardiac disease. Cardiac work-up demonstrated aortic stenosis, mitral stenosis, and multivessel coronary artery disease requiring aortic valve replacement, mitral valve replacement, and coronary artery bypass grafting. During the operation, significant discoloration of tissue was observed. This correlated with areas of severe calcification, which was noted throughout both valves. Extensive debridement was required prior to proceeding to valve replacements. Additionally, near-infrared spectroscopy failed to provide accurate measurements of cerebral oxygenation. Discussion Alkaptonuria is correlated with cardiovascular disease, particularly valvular disease. Intraoperatively, these patients may exhibit noticeable discoloration and severe calcification of various tissues. Additionally, traditional infrared-based methods of cerebral oxygenation monitoring may not be reliable; however, other options of cerebral monitoring may be feasible. With proper pre-operative planning, however, patients with alkaptonuria may safely undergo cardiac surgery.
Liposarcomas are the most common soft tissue tumors, occurring predominantly in the lower limbs and retroperitoneum.Primary mediastinal liposarcomas are rare and account for less than 1% of mediastinal masses.We report a case of a large primary mediastinal liposarcoma invading the right ventricular outflow tract, pulmonary artery, and aortic adventitia, which was successfully resected.This report highlights the complex nature of liposarcomas given their tendency to invade critical structures.
The purpose of our study was to assess the value of true lumen and false lumen hemodynamics compared to aortic morphological measurements for predicting adverse-aorta related outcomes (AARO) and aortic growth in patients with type B aortic dissection (TBAD).Using an IRB approved protocol, we retrospectively identified patients with descending aorta (DAo) dissection at a large tertiary center. Inclusion criteria includes known TBAD with ≥ 6 months of clinical follow-up after initial presentation for TBAD or after ascending aorta intervention for patients with repaired type A dissection with residual type B aortic dissection (rTAAD). Patients with prior descending aorta intervention were excluded. The FL and TL of each patient were manually segmented from 4D flow MRI data, and 3D parametric maps of aortic hemodynamics were generated. Groups were divided based on (1) presence vs. absence of AARO and (2) growth rate ≥ vs. < 3 mm/year. True and false lumen kinetic energy (KE), stasis, peak velocity (PV), reverse/forward flow (RF/FF), FL to TL KE ratio, as well as index aortic diameter were compared between groups using the Mann-Whitney U or independent t-test.A total of n = 51 patients (age: 58.4 ± 15.0 years, M/F: 31/20) were included for analysis of AARO. This group contained n = 26 patients with TBAD and n = 25 patients with rTAAD. In the overall cohort, AARO patients had larger baseline diameters, lower FL-RF, FL stasis, TL-KE, TL-FF and TL-PV. Among patients with de novo TBAD, those with AAROs had larger baseline diameter, lower FL stasis and TL-PV. In both the overall cohort and in the subgroup of de novo TBAD, subjects with aortic growth ≥ 3mm/year, patients had a higher KE ratio.Our study suggests that 4D flow MRI is a promising tool for TBAD evaluation that can provide information beyond traditional MRA or CTA. 4D flow has the potential to become an integral aspect of TBAD work-up, as hemodynamic assessment may allow earlier identification of at-risk patients who could benefit from earlier intervention.
